Please use this identifier to cite or link to this item:
|Scopus||Web of Science®||Altmetric|
|Title:||Paranodal pathology in Tangier disease with remitting-relapsing multifocal neuropathy|
|Citation:||Journal of Clinical Neuroscience, 2006; 13(4):492-497|
|Z. Cai, P.C. Blumbergs, K. Cash, P.J. Rice, J. Manavis, J. Swift, Mounir N. Ghabriel and P.D. Thompson|
|Abstract:||Pathological studies of a sural nerve biopsy in a man with Tangier disease presenting as a remitting-relapsing multifocal neuropathy showed abnormalities in the paranodal regions, including lipid deposition (65%) and redundant myelin foldings, with various degrees of myelin splitting and vesiculation (43%) forming small tomacula and abnormal myelin terminal loops (4%). The internodal regions were normal in the majority of myelinated fibres. Abnormal lipid storage was also present in the Schwann cells of the majority of unmyelinated fibres (67%). The evidence suggests that the noncompacted myelin region of the paranode is a preferential site for lipid storage in the myelinated Schwann cell, and that the space-occupying effects of the cholesterol esters leads to paranodal malfunction and tomacula formation as the pathological basis for the multifocal relapsing-remitting clinical course.|
|Keywords:||Tangier disease; Remitting-relapsing neuropathy; Paranode|
|Description:||Copyright © 2006 Elsevier Ltd All rights reserved.|
|Appears in Collections:||Medicine publications|
Files in This Item:
There are no files associated with this item.
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.